Bilateral Retinoblastoma: Management and Impact on the Lives of Survivors

Abstract

Introduction: Retinoblastoma is a malignant tumor of neuroepithelial origin occurring in infants and young children. Despite its rarity, it is the most common intraocular malignancy in children. It is unilateral in 2/3 of cases and bilateral in 1/3, and may represent a genetic predisposition to cancer. RB survivors are at risk of disease- and treatment-related adverse effects, including loss of vision or one eye, second cancer, genetic predisposition to cancer and reduced quality of life. The aim of our study is to investigate the management Bilateral Retinoblastoma and its impact on the lives of survivors.

Method: We report in this retrospective study of 15 cases collected at the Department of Hematology and Pediatric Oncology at CHU Mohammed VI in Marrakech over a 12-year period, from January 2012 to June 2O24.

Result: The mean age was 1 year and 5 months with a slight male predominance (60%). No patients had a family history of retinoblastoma. Consanguinity was present in 26%. The most frequent reasons for consultation were: leukocoria (86.6%), strabismus (33.3%) and exophthalmos (13.33%). The majority of patients (80%) were seen within 6 to 14 months, with an average delay of 9 months. Diagnosis was based on ophthalmoscopic examination, ultrasound and CT scan data, while histological examination of the surgical specimen enabled confirmation of the diagnosis and prognostic classification. Optic nerve invasion was found in 3 cases, and choroidal involvement in 3 patients. Therapeutically, enucleation was required in most cases, given the advanced stage of the tumours (86.66%): enucleation of the right eye in 4 patients, enucleation of the left eye in 2 patients, and bilateral enucleation in 8 patients, given the advanced stage of the tumours. Neoadjuvant chemotherapy was indicated in 46.6% of cases, to facilitate enucleation. Eleven 11 patients (73.3%) received post-operative chemotherapy, 3 patients received unilateral conservative treatment (thermotherapy, cryotherapy and intravitreal chemotherapy) and 1 patient received bilateral conservative treatment (radiotherapy).

In our series, 11 patients achieved complete remission, two died of severe bone marrow failure after chemotherapy, 1 was lost to follow-up and one girl died of osteosarcoma of the left shoulder. Only one case of tumor recurrence in the optic disc was noted, subsequently put on palliative treatment, 72% benefited from an ocular prosthesis.

The parents of the survivors in our study perceived their children's general health and emotional quality of life as inferior (70%), and the participation of children who had undergone unilateral enucleation in school and the community was significantly higher than that of children who had undergone bilateral enucleation.

Conclusion: Enucleation and chemotherapy remains the gold standard in the management of bilateral retinoblastoma. Enucleation continues to encounter a range of psychosocial problems.

Lifelong follow-up and advice on a healthy lifestyle are necessary for retinoblastoma survivors.